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Ten‐year survivors in AL amyloidosis: characteristics and treatment pattern

Published on Dec 1, 2019in British Journal of Haematology5.206
· DOI :10.1111/bjh.16096
Eli Muchtar11
Estimated H-index: 11
(Mayo Clinic),
Morie A. Gertz103
Estimated H-index: 103
(Mayo Clinic)
+ 22 AuthorsAngela Dispenzieri97
Estimated H-index: 97
(Mayo Clinic)
Abstract
  • References (18)
  • Citations (1)
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References18
Newest
#1Eli Muchtar (Mayo Clinic)H-Index: 11
#2Terry M. Therneau (Mayo Clinic)H-Index: 98
Last. Angela Dispenzieri (Mayo Clinic)H-Index: 97
view all 25 authors...
3 CitationsSource
#1Giampaolo Merlini (UNIPV: University of Pavia)H-Index: 78
#2Angela Dispenzieri (Mayo Clinic)H-Index: 97
Last. Morie A. Gertz (Mayo Clinic)H-Index: 103
view all 7 authors...
Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. The disease is progressive and, accordingly, early diagnosis is vital to prevent irreversible organ damage, of which cardiac damage and renal damage predominate. The development of novel sensitive biomarkers and imaging technologies for the det...
29 CitationsSource
#1Arjun Lakshman (Mayo Clinic)H-Index: 5
#2Shilpa Paul (Mayo Clinic)H-Index: 1
Last. Shaji K. Kumar (Mayo Clinic)H-Index: 76
view all 26 authors...
7 CitationsSource
#1Sajitha Sachchithanantham (UCL: University College London)H-Index: 12
#2O. BerlangaH-Index: 1
Last. Ashutosh D. Wechalekar (UCL: University College London)H-Index: 38
view all 9 authors...
Summary Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. HLC measurements identified immunoparesis of at least one immunoglo...
5 CitationsSource
#1Luis Gerardo Rodríguez-Lobato (University of Barcelona)H-Index: 3
#2C. Fernández de Larrea (University of Barcelona)H-Index: 16
Last. J. Bladé (University of Barcelona)H-Index: 84
view all 11 authors...
AbstractObjectives: Immunoparesis (IP) is a risk factor associated with an unfavourable outcome in several plasma cell disorders. It has been suggested that its presence in light-chain (AL) amyloidosis could be associated with worse prognosis. However, the relevance of IP after treatment has not been evaluated to date. The aim of this study was to determine the prognostic impact of IP at diagnosis and one year after treatment onset in patients with AL amyloidosis.Methods: The clinical records of...
2 CitationsSource
#1Eli Muchtar (Mayo Clinic)H-Index: 11
#2Angela Dispenzieri (Mayo Clinic)H-Index: 97
Last. Morie A. Gertz (Mayo Clinic)H-Index: 103
view all 22 authors...
Interphase fluorescence in situ hybridization in untreated AL amyloidosis has an independent prognostic impact by abnormality type and treatment category
21 CitationsSource
#1Eli MuchtarH-Index: 11
#2Morie A. GertzH-Index: 14
Last. Angela DispenzieriH-Index: 97
view all 24 authors...
In light of major advances in immunoglobulin light chain (AL) amyloidosis, we evaluated the trends in presentation, management, and outcome among 1551 newly diagnosed AL amyloidosis patients seen in our institution from 2000 to 2014. As compared with the 2 intervals 2000-2004 and 2005-2009, patients diagnosed in 2010-2014 were less likely to have >2 involved organs. Utilization of autologous stem cell transplant (ASCT) was similar across all periods, about one-third of patients, but there was an...
76 CitationsSource
#1Eli Muchtar (Mayo Clinic)H-Index: 11
#2Angela Dispenzieri (Mayo Clinic)H-Index: 97
Last. Morie A. Gertz (Mayo Clinic)H-Index: 103
view all 20 authors...
Immunoparesis is an adverse prognostic marker in plasma cell proliferative disorders. Its impact in AL amyloidosis has not been explored in depth. Newly diagnosed AL amyloidosis patients (n=998) were evaluated for immunoparesis by two methods. The first method was qualitative, considering the number of suppressed uninvolved immunoglobulins below the lower limit of normal (LLN) (none, partial, all). The second method was quantitative, assessing the average relative difference (ARD) of the uninvol...
12 CitationsSource
#1Eli Muchtar (Mayo Clinic)H-Index: 11
#2Angela Dispenzieri (Mayo Clinic)H-Index: 97
Last. Morie A. Gertz (Mayo Clinic)H-Index: 103
view all 18 authors...
Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglobulins, classifying patients into a negative average relative difference (i.e. significant immunoparesi...
5 CitationsSource
#1Eli Muchtar (TAU: Tel Aviv University)H-Index: 11
#2Hila Magen (TAU: Tel Aviv University)H-Index: 5
Last. Pia Raanani (TAU: Tel Aviv University)H-Index: 29
view all 9 authors...
Abstract Immunoparesis serves as a marker for elevated risk for progression in plasma cell proliferative disorders. However, the impact of immunoparesis in AL amyloidosis has not been addressed. Immunoparesis was defined qualitatively as any decrease below the low reference levels of the uninvolved immunoglobulins and quantitatively, as the relative difference between the uninvolved immunoglobulins and the lower reference values. Forty-one newly diagnosed AL amyloidosis patients were included. S...
5 CitationsSource
Cited By1
Newest
#1Eli Muchtar (Mayo Clinic)H-Index: 11
#2Grace Lin (Mayo Clinic)H-Index: 24
Last. Martha Grogan (Mayo Clinic)H-Index: 32
view all 3 authors...
Abstract Light chain (AL) amyloidosis is a systemic syndrome characterized by progressive organ dysfunction leading to organ failure and death. The heart is the most commonly involved organ and the leading determinant of short and long-term survival. Pathogenic free light chains, fragments of intact immunoglobulins, are the amyloidogenic protein and are secreted by clonal bone marrow plasma cells. The goal of therapy is to cut-off the supply of these pathogenic light chains to allow organ recove...
1 CitationsSource
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